Review Articles
Determinants of quality of life in children and adolescents with CHD: a systematic review
- Maria Drakouli, Konstantinos Petsios, Margarita Giannakopoulou, Elisabeth Patiraki, Ioanna Voutoufianaki, Vasiliki Matziou
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- Published online by Cambridge University Press:
- 16 February 2015, pp. 1027-1036
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Purpose
This review aims to outline a systematic approach for the assessment of quality of life in children and adolescents with CHD and to cite its main determinants.
MethodsA systematic critical literature search in PubMed, Scopus, and Cinahl databases resulted in 954 papers published after 2000. After the quality assessment, 32 original articles met the inclusion criteria.
ResultsMethodological quality of the included studies varied greatly, showing a moderate quality. Impaired quality of life was associated with more severe cardiac lesions. Children with CHD, after cardiac surgery, reported diminished quality of life concerning physical, psycho-social, emotional, and school functioning. The majority of clinical studies showed significant differences among children and their parents’ responses regarding their quality of life, with a tendency of children to report greater quality of life scores than their parents. According to our analysis, concerning children with CHD, the most cited determinants of their quality of life were as follows: (a) parental support; (b) lower socio-economic status; (c) limitations due to physical impairment; (d) sense of coherence; as well as (e) the level of child’s everyday anxiety and depression. These findings suggest that differences in quality of life issues may exist across lesion severities.
ConclusionQuality of life in children with CHD should be assessed according to age; severity; therapeutic approach; acceptance of the disease; and personality features. Effective management and early recognition of significant impairments in quality of life could impact clinical outcomes in children with CHD.
Isomerism or heterotaxy: which term leads to better understanding?
- Rohit S. Loomba, Anthony M. Hlavacek, Diane E. Spicer, Robert H. Anderson
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- 19 June 2015, pp. 1037-1043
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Use of correct nomenclature is important in all aspects of medicine. Many of the controversies that have bedeviled paediatric cardiology have devolved from the inappropriate use of words to describe the lesions to be found when the heart is congenitally abnormal. A continuing area of disagreement is the situation currently described by many as representing “heterotaxy”. When used literally, this word means any departure from the normal. Thus, all congenitally malformed hearts represent examples of heterotaxy. By convention, nonetheless, the term is used to describe the arrangement in which the bodily organs, including parts of the heart, are not in their usual or in their mirror-imaged patterns. The arrangements, therefore, represent the presence of the organs on the right and left sides of the body being mirror imaged, in other words isomeric; however, not all the organs are uniformly isomeric. In this review, we show that, when assessed on the basis of the morphology of the atrial appendages, specifically the extent of the pectinate muscles relative to the atrioventricular junctions, isomerism is an unequivocal finding within the heart. Only the atrial appendages, however, are truly isomeric. The potential problem of disharmony between the various systems of organs is resolved simply by accounting specifically for each of the systems. On these bases, we suggest that the isomeric arrangements can now readily be diagnosed in the clinical setting, and differentiated into their right and left isomeric variants. We propose that such distinctions will provide the key for establishing the genetic cues responsible for the formation of the isomeric as opposed to the lateralised arrangements.
Original Articles
Childhood growth patterns following congenital heart disease
- David C. Aguilar, Gary W. Raff, Daniel J. Tancredi, Ian J. Griffin
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- 23 September 2014, pp. 1044-1053
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Introduction: Prenatal and early postnatal growth are known to be abnormal in patients with CHD. Although adult metabolic risk is associated with growth later in childhood, little is known of childhood growth in CHD. Patients and Methods: Retrospective data were collected on 551 patients with coarctation of the aorta, hypoplastic left heart syndrome, single ventricle physiology, tetralogy of Fallot, transposition of the great arteries, or ventricular septal defects. Weight, height, and body mass index data were converted to Z-scores. Body size at 2 years and growth between 2 and 20 years, 2 and 7 years, and 8 and 15 years were compared with Normative data using a sequential series of mixed-effects linear models. Results: A total of 4660 weight, 2989 height, and 2988 body mass index measurements were analysed. Body size at 2 years of age was affected by cardiac diagnosis and gender. Abnormal growth was frequent and varied depending on cardiac diagnosis, gender, and the time period considered. The most abnormal patterns were seen in patients with tetralogy of Fallot, hypoplastic left heart syndrome, or single ventricle physiology. Potentially high-risk growth, a combination of small body size at 2 years and rapid subsequent growth, was seen in several groups. Conclusions: Childhood and adolescent growth patterns were gender- and lesion-specific. Several lesions were associated with abnormal patterns of childhood growth known to be associated with an increased risk of adult adiposity or metabolic risk in other populations. Further information is needed on the long-term metabolic risks of survivors of CHD.
Proteinuria and clinical outcome in CHD patients
- Efrén Martínez-Quintana, Fayna Rodríguez-González
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- 28 August 2014, pp. 1054-1059
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Introduction: CHD patients, especially those with associated hypoxaemia, usually have some level of renal function impairment, even though they are relatively young. The aim of the study was to evaluate those clinical and analytical factors that may contribute to microalbuminuria and determine the association of 24-hour proteinuria with thrombotic events and mortality. Methods: A total of 251 CHD patients were studied and demographic characteristics, blood test, and 24-hour urinalysis were analysed. Results: Of the patients, 221 were non-hypoxaemic, and 30 were hypoxaemic (oxygen saturation of 84.3±5.9%). Of the non-hypoxaemic patients, 30 (13.6%), and of the hypoxaemic patients 9 (30%), showed proteinuria (>0.15 g/24 hours) (p=0.028). Hypoxaemic CHD patients also showed higher haematocrit (%) (50.7 (34.6; 72.1) versus 42.8 (34.6; 48.9), p<0.001), serum creatinine (mg/dl) (1.07±0.2 versus 0.96±1.9, p=0.004), microalbuminuria (mg/dl/24 hours) (1.2 (0.0; 261.5) versus 0.5 (0.0; 4.37), p<0.001), proteinuria (gr/24 hours) (1.0 (0.4; 3.1) versus 0.08 (0.04; 0.52), p=0.043), and N-terminal pro–B-type natriuretic peptide (pg/ml) (417.8 (35.7; 8534.0) versus 44.9 (0.0; 670.5), p<0.001) concentrations than non-hypoxaemic CHD patients. During a median follow-up of 26.0 (16.9; 57.7) months, five patients died – one patient had 24-hour proteinuria and four patients did not (p=0.581) – and three patients had some type of thrombosis – two patients had 24-hour proteinuria and one patient did not (p=0.014). Kaplan–Meier survival analysis showed no significant difference between CHD patients with and without 24-hour proteinuria (p=0.631). Conclusion: CHD patients with proteinuria have significantly more thrombosis and more hypoxaemia than those patients without proteinuria.
Intravenous paracetamol with a lower dose is also effective for the treatment of patent ductus arteriosus in pre-term infants
- Kadir Şerafettin Tekgündüz, Naci Ceviz, İbrahim Caner, Haşim Olgun, Yaşar Demirelli, Canan Yolcu, İrfan Oğuz Şahin, Mustafa Kara
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- 27 August 2014, pp. 1060-1064
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Introduction: Haemodynamically significant patent ductus arteriosus is a significant cause of morbidity and mortality in pre-term infants. This retrospective study was conducted to investigate the usefulness of lower-dose paracetamol for the treatment of patent ductus arteriosus in pre-term infants. Materials and Methods: A total of 13 pre-term infants who received intravenous paracetamol because of contrindications or side effects to oral ibuprofen were retrospectively enrolled. In the first patient, the dose regimen was 15 mg/kg/dose, every 6 hours. As the patient developed significant elevation in transaminase levels, the dose was decreased to 10 mg/kg/dose, every 8 hours in the following 12 patients. Echocardiographic examination was conducted daily. In case of closure, it was repeated after 2 days and when needed thereafter in terms of reopening. Results: A total of 13 patients received intravenous paracetamol. Median gestational age was 29 weeks ranging from 24 to 31 weeks and birth weight was 950 g ranging from 470 to 1390 g. The median postnatal age at the first intravenous paracetamol dose was 3 days ranging from 2 to 9 days. In 10 of the 13 patients (76.9%), patent ductus arteriosus was closed at the median 2nd day of intravenous paracetamol ranging from 1 to 4 days. When the patient who developed hepatotoxicity was eliminated, the closure rate was found to be 83.3% (10/12). Conclusion: Intravenous paracetamol may be a useful treatment option for the treatment of patent ductus arteriosus in pre-term infants with contrindication to ibuprofen. In our experience, lower-dose paracetamol is effective in closing the patent ductus arteriosus in 83.3% of the cases.
Psychosocial responses of parents to their infant’s diagnosis of hypoplastic left heart syndrome
- Annie M. Cantwell-Bartl, James Tibballs
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- 12 September 2014, pp. 1065-1073
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Objective: To evaluate the psychosocial status of mothers and fathers in response to their infant’s diagnosis of hypoplastic left heart syndrome. Design: A study on interviews with parents whose children had survived staged surgery. Setting: Tertiary hospital paediatric ICU. Subjects: A total of 29 parents (16 mothers and 13 fathers) of surviving children. Intervention: A semi-structured face-to-face interview was conducted to explore experiences of parents in response to their infant’s diagnosis, their interaction with the doctor delivering the diagnosis, their deliberation about staged surgery, and their reasons for this choice. Measurement and Main Results: All parents were devastated about their infant’s diagnosis, and most (83%) of them said that the time of the diagnosis and the aftermath was the worst time of their lives. Parents reported helpful and unhelpful communication at this time. Although all parents in this study chose surgery for their infant, when faced with the choice, 17 of them made an immediate decision “to protect their infant’s life”, 8 were initially unsure when their infant was diagnosed in utero, and 4 were unsure when the infant was diagnosed after birth. Parents also experienced loss and other stressors. Conclusions: All parents of the infants diagnosed with hypoplastic left heart syndrome experienced intense loss and stressors. Physicians need to be sensitive to the needs and thinking of the parents when discussing treatment options before surgery. The nature of the relationship with the doctor at this time can support parents or be a further source of stress.
Ultrasound assessment of mesenteric blood flow in neonates with hypoplastic left heart before and after hybrid palliation
- Corin T. Cozzi, Mark Galantowicz, John P. Cheatham, Lisa Nicholson, Richard Fernandez, Carl H. Backes, Carrie McCaw, Clifford L. Cua
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- 12 September 2014, pp. 1074-1079
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Background: Altered mesenteric perfusion may be a contributor to the development of necrotising enterocolitis in patients with hypoplastic left heart syndrome. The goal of this study was to document mesenteric flow patterns in patients with hypoplastic left heart syndrome pre- and post-hybrid procedure. Methods: A prospective study on all patients with hypoplatic left heart syndrome undergoing the hybrid procedure was conducted. Doppler ultrasound analysis of the coeliac and superior mesenteric artery was performed. Results: A total of 13 patients were evaluated. There was a significant difference in the coeliac artery effective velocity-time intergral pre- and post-hybrid procedure (8.69±3.84 versus 12.51±4.95 cm, respectively). There were significant differences in the superior mesenteric artery antegrade velocity-time integral pre- and post-hybrid procedure (6.86±2.45 versus 10.52±2.64 cm, respectively) and superior mesenteric artery effective velocity-time integral pre- and post-hybrid procedure (6.22±2.68 versus 9.73±2.73 cm, respectively). There were no significant differences between the coeliac and superior mesenteric artery Doppler indices in the pre-hybrid procedure; there were, however, significant differences in the post-hybrid procedure between coeliac and superior mesenteric artery antegrade velocity-time integral (13.8 2±5.60 versus 10.52±2.64 cm, respectively) and effective velocity-time integral (13.04±4.71 versus 9.73±2.73 cm, respectively). Conclusion: Doppler mesenteric indices of perfusion improve in patients with hypoplastic left heart syndrome after the hybrid procedure; however, there appears to be preferential flow to the coeliac artery versus the superior mesenteric artery in these patients post-procedure.
Efficacy of digoxin in comparison with propranolol for treatment of infant supraventricular tachycardia: analysis of a large, national database
- Brady S. Moffett, Philip J. Lupo, Caridad M. delaUz, Santiago O. Valdes, Christina Y. Miyake, Jamie A. Decker, Jeffrey J. Kim
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- 12 September 2014, pp. 1080-1085
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Introduction: Digoxin or propranolol are used as first-line enteral agents for treatment of infant supraventricular tachycardia. We used a large national database to determine whether enteral digoxin or propranolol was more effciacious as first-line infant supraventricular tachycardia therapy. Materials and Methods: The Pediatric Health Information System database was queried over 10 years for infants with supraventricular tachycardia initiated on enteral digoxin or propranolol monotherapy. Patients were excluded for Wolff–Parkinson–White, intravenous antiarrhythmics (other than adenosine), or death. Success was considered as discharge on the initiated monotherapy. Risk factors for successful monotherapy and risk factors for readmission for supraventricular tachycardia for patients discharged on monotherapy were determined. Results: A total of 374 patients (59.6% male) met the study criteria. Median length of stay was 7 days (interquartile range of 3–16 days). Patients had CHD (n=199, 53.2%) and underwent cardiac surgery (n=123, 32.9%), ICU admission (n=238, 63.6%), mechanical ventilation (n=146, 39.0%), and extracorporeal membrane oxygenation (n=3, 0.8%). Pharmacotherapy initiation was at median 37 days of life (interquartile range of 12–127 days) and 47.3% were initiated on digoxin. Success was similar between digoxin (73.1%) and propranolol (73.5%). Initial therapy with digoxin was not associated with success (odds ratio 1.01, 95% CI 0.64–1.61, p=0.93). Multivariable analysis demonstrated hospital length of stay (odds ratio 0.98, 95% CI 0.98–1.00) and involvement of a paediatric cardiologist (odds ratio 0.46, 95% CI 0.29–0.75) associated with monotherapy failure, and male gender (odds ratio 1.66, 95% CI 1.03–2.67) associated with monotherapy success. No variables were significant for readmission on multivariable analysis. Discussion: Digoxin or propranolol may be equally efficacious for inpatient treatment of infant supraventricular tachycardia.
Relationship of birth weight with congenital cardiovascular malformations in a population-based study
- Robert A. Petrossian, Karen S. Kuehl, Christopher A. Loffredo
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- 28 August 2014, pp. 1086-1092
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Introduction: A known comorbidity of congenital cardiovascular malformations is low birth weight, but the reasons for this association remain obscure. This retrospective study examines the relationship between congenital cardiovascular malformations and the birth weight of singletons, taking into account differences in gestational age and other factors. Methods: Using data from the retrospective, population-based Baltimore–Washington Infant Study, six types of congenital cardiovascular malformations were investigated in comparison with controls (n=3519) through linear regression: d-transposition of the great arteries (n=187), other conotruncal heart defects (n=361), endocardial cushion defects (n=281), left heart obstructive lesions (n=507), atrial septal defects (n=281), and membranous ventricular septal defects (n=622). Results: Infants with conotruncal heart defects (−218 g), endocardial cushion defects with Down syndrome (−265 g), endocardial cushion defects without Down syndrome (−194 g), left heart obstructive lesions (−143 g), atrial septal defects (−150 g), and membranous ventricular septal defects (−127 g) showed significant birth weight deficits, adjusting for gestational age, and other covariates. Infants with d-transposition of the great arteries (−67 g) did not show significant birth weight deficits compared with the control group. Discussion: The degree of birth weight decrement appears to be highly related to the specific type of congenital cardiovascular malformation. As a whole, these infants do not exhibit low birth weights solely because of being premature, and thus other mechanisms must underlie these associations.
Clinical and molecular characterisation of Holt–Oram syndrome focusing on cardiac manifestations
- Won Kyoung Jhang, Beom Hee Lee, Gu-Hwan Kim, Jin-Ok Lee, Han-Wook Yoo
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- 12 September 2014, pp. 1093-1098
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Background: Holt–Oram syndrome is characterised by CHD and limb anomalies. Mutations in TBX5 gene, encoding the T-box transcription factor, are responsible for the development of Holt–Oram syndrome, but such mutations are variably detected in 30–75% of patients. Methods: Clinically diagnosed eight Holt–Oram syndrome patients from six families were evaluated the clinical characteristics, focusing on the cardiac manifestations, in particular, and molecular aetiologies. In addition to the investigation of the mutation of TBX5, SALL4, NKX2.5, and GATA4 genes, which are known to regulate cardiac development by physically and functionally interacting with TBX5, were also analyzed. Multiple ligation-dependent probe amplification analysis was performed to detect exonic deletion and duplication mutations in these genes. Results: All included patients showed cardiac septal defects and upper-limb anomalies. Of the eight patients, seven underwent cardiac surgery, and four suffered from conduction abnormalities such as severe sinus bradycardia and complete atrioventricular block. Although our patients showed typical clinical findings of Holt–Oram syndrome, only three distinct TBX5 mutations were detected in three families: one nonsense, one splicing, and one missense mutation. No new mutations were identified by testing SALL4, NKX2.5, and GATA4 genes. Conclusions: All Holt–Oram syndrome patients in this study showed cardiac septal anomalies. Half of them showed TBX5 gene mutations. To understand the genetic causes for inherited CHD such as Holt–Oram syndrome is helpful to take care of the patients and their families. Further efforts with large-scale genomic research are required to identify genes responsible for cardiac manifestations or genotype–phenotype relation in Holt–Oram syndrome.
Holes and channels between the ventricles revisited
- Adrian Crucean, William J. Brawn, Diane E. Spicer, Rodney C. Franklin, Robert H. Anderson
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- 23 September 2014, pp. 1099-1110
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Background
Although holes, or channels, between the ventricles are the commonest congenital cardiac malformations, there is still no consensus as to how they can best be described and categorised. So as to assess whether it is possible to produce a potentially universally acceptable system, we have analysed the hearts categorised as having ventricular septal defects in a large archive held at Birmingham Children’s Hospital.
Materials and methodsWe analysed all the hearts categorised as having isolated ventricular septal defects, or those associated with aortic coarctation or interruption in the setting of concordant ventriculo-arterial connections, in the archive of autopsied hearts held at Birmingham Children’s Hospital, United Kingdom.
ResultsWe found 147 hearts within the archive fulfilling our criterions for inclusion. All could be classified within one of three groups depending on their borders as seen from the right ventricle. To provide full description, however, it was also necessary to take account of the way the defects opened to the right ventricle, and the presence or absence of alignment between the septal components.
ConclusionsBy combining information on the phenotypic specificity defined on the basis of their borders, the direction of opening into the right ventricle, and the presence or absence of septal malalignment, it proved possible to categorise all hearts examined within the archive of Birmingham Children’s Hospital. Our findings have necessitated creation of new numbers within the European Paediatric Cardiac Code.
Follow-up of congenital heart disease patients with subclinical hypothyroidism
- Efrén Martínez-Quintana, Fayna Rodríguez-González
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- 23 September 2014, pp. 1111-1118
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Introduction: Subclinical hypothyroidism or mild thyroid failure is a common problem in patients without known thyroid disease. Methods: Demographic and analytical data were collected in 309, of which 181 were male and 128 were female, congenital heart disease (CHD) patients. CHD patients with thyroid-stimulating hormone above 5.5 mIU/L were also followed up from an analytical point of view to determine changes in serum glucose, cholesterol, N-terminal pro b-type natriuretic peptide, and C-reactive protein concentrations. Results: Of the CHD patients, 35 (11.3%) showed thyroid-stimulating hormone concentration above 5.5 mIU/L. Of them, 27 were followed up during 2.4±1.2 years – 10 were under thyroid hormone replacement treatment, and 17 were not. Of the 27 patients (25.9%), 7 with subclinical hypothyroidism had positive anti-thyroid peroxidase, and 3 of them (42.8%) with positive anti-thyroid peroxidase had Down syndrome. Down syndrome and hypoxaemic CHD patients showed higher thyroid-stimulating hormone concentrations than the rest of the congenital patients (p<0.001). No significant differences were observed in serum thyroxine, creatinine, uric acid, lipids, C-reactive protein, or N-terminal pro b-type natriuretic peptide concentrations before and after the follow-up in those CHD patients with thyroid-stimulating hormone above 5.5 mIU/L whether or not they received levothyroxine therapy. Conclusions: CHD patients with subclinical hypothyroidism showed no significant changes in serum thyroxine, cholesterol, C-reactive protein, or N-terminal pro b-type natriuretic peptide concentrations whether or not they were treated with thyroid hormone replacement therapy.
Surgical management of symptomatic neonates with Ebstein’s anomaly: choice of operation
- Christopher J. Knott-Craig, Thittamaranahalli Kariyappa S. Kumar, Alejandro R. Arevalo, Vijaya M. Joshi
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- 24 September 2014, pp. 1119-1123
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Objective: Symptomatic neonates with Ebstein’s anomaly pose significant challenge. Within this cohort, neonates with associated anatomical pulmonary atresia have higher mortality. We review our experience with this difficult subset. Methods: A total of 32 consecutive symptomatic neonates with Ebstein’s anomaly underwent surgical intervention between 1994 and 2013. Of them, 20 neonates (62%, 20/32) had associated pulmonary atresia. Patients’ weights ranged from 1.9 to 3.4 kg. All patients without pulmonary atresia had two-ventricle repair. Of the 20 neonates, 16 (80%, 16/20) with Ebstein’s anomaly and pulmonary atresia had two-ventricle repair and 4 had single-ventricle palliation, of which 2 underwent Starnes’ palliation and 2 Blalock–Taussig shunts. Six recent patients with Ebstein’s anomaly and pulmonary atresia had right ventricle to pulmonary artery valved conduit as part of their two-ventricle repair. Results: Overall early mortality was 28% (9/32). For those without pulmonary atresia, mortality was 8.3% (1/12). For the entire cohort of neonates with Ebstein’s anomaly and pulmonary atresia, mortality was 40% (8/20; p=0.05). Mortality for neonates with Ebstein’s anomaly and pulmonary atresia having two-ventricle repair was 44% (7/16). Mortality for neonates with Ebstein’s anomaly and pulmonary atresia having two-ventricle repair utilising right ventricle to pulmonary artery conduit was 16% (1/6). For those having one-ventricle repair, the mortality was 25% (1/4). Conclusions: Surgical management of neonates with Ebstein’s anomaly remains challenging. For neonates with Ebstein’s anomaly and anatomical pulmonary atresia, single-ventricle palliation is associated with lower early mortality compared with two-ventricle repair. This outcome advantage is negated by inclusion of right ventricle to pulmonary artery conduit as part of the two-ventricle repair.
Prevalence and predictors of coronary artery disease in adults with Kawasaki disease
- Jalaj Garg, Parasuram Krishnamoorthy, Chandrasekar Palaniswamy, Rajiv Paudel, Saurav Chatterjee, Hasan Ahmad, Christopher Snyder
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- 23 October 2014, pp. 1124-1129
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Background: Accelerated coronary atherosclerosis in patients with Kawasaki disease, in conjunction with coronary artery aneurysm and stenosis that characterise this disease, are potential risk factors for developing coronary artery disease in young adults. We aimed to determine the prevalence and predictors of coronary artery disease in adult patients with Kawasaki disease. Methods: All patients aged 18−55 years of age diagnosed with Kawasaki disease were sampled from Nationwide Inpatient Sample database using International Classification of Diseases 9th revision (ICD 9 code 446.1) from 2009 to 2010. Demographics, prevalence of coronary artery disease, and other traditional risk factors in adult patients with Kawasaki disease were analysed using ICD 9 codes. Results: The prevalence of Kawasaki disease among adults was 0.0005% (n=215) of all in-hospital admissions in United States. The mean age was 27.3 years with women (27.6 years) older than men (27.1 years). Traditional risk factors were hypertension (21%), hyperlipidaemia (15.6%), diabetes (11.5%), tobacco use (8.8%), and obesity (8.8%), with no significant difference between men and women. Coronary artery disease (32.4%), however, was more prevalent in men (44.7%) than in women (12.1%; p=0.03). In multivariate regression analysis, after adjusting for demographics and traditional risk factors, hypertension (OR=13.2, p=0.03) was an independent risk factor of coronary artery disease. Conclusion: There was increased preponderance of coronary artery disease in men with Kawasaki disease. On multivariate analysis, hypertension was found to be the only independent predictor of coronary artery disease in this population after adjusting for other risk factors.
Diastolic function in anthracycline-treated children
- Ashraf Harahsheh, Sanjeev Aggarwal, Michael D. Pettersen, Thomas L’Ecuyer
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- 23 September 2014, pp. 1130-1135
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Background: Anthracyclines are effective medications for childhood cancer. Their limitation is the risk of cardiomyopathy. Although diastolic dysfunction has been described in patients who received anthracyclines, cardiac monitoring has focused on systolic function, which is abnormal in up to 41% of the patients. We conducted a study to assess diastolic function utilising transmitral inflow Doppler velocities and tissue Doppler imaging in anthracycline-treated children 5 years post-therapy. Methods: This was a retrospective study on 63 anthracycline-treated patients. Echocardiographic parameters included peak early and late transmitral inflow Doppler velocities (E, A), E/A ratio, E deceleration time, and tissue Doppler imaging early and late diastolic mitral annulus velocities (E′, A′), E/E′ ratio, and E′/A′ ratio. Results: All indices of diastolic function that we measured were normal in the anthracycline-treated patients. Conclusion: We conclude that diastolic function assessed by transmitral inflow Doppler velocities and tissue Doppler imaging is normal in anthracycline-treated children 5 years after completion of treatment. Further longitudinal study is needed to determine whether diastolic function becomes abnormal with time in this patient population.
Pulmonary vasodilator therapy and early postoperative outcome after modified Fontan operation
- Alberto Mendoza, Leticia Albert, Sylvia Belda, Lidia Casanueva, Dolores Herrera, Miguel A. Granados, José M. Velasco, Enrique García, Juan M. Aguilar, Juan V. Comas
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- 16 September 2014, pp. 1136-1140
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Although mortality is low after the modified Fontan procedure, there is a significant percentage of patients with prolonged postoperative recovery. The objective of this study is to evaluate the usefulness of postoperative administration of oral sildenafil and inhaled nitric oxide on early postoperative outcome.
A prospective interventional and comparison study with a historical cohort was conducted. Between January, 2010 and March, 2013, 16 patients received oral sildenafil during immediate modified Fontan postoperative period. Inhaled nitric oxide was also administered if the patient was kept intubated 12 hours after surgery. Early postoperative outcome was compared with a historical cohort of 32 patients on whom the modified Fontan procedure was performed between March, 2000 and December, 2009.
Postoperative administration of sildenafil and nitric oxide had no influence on early postoperative outcome after the modified Fontan procedure in terms of duration of pleural effusions, mechanical ventilation time, length of stay in the ICU, and length of hospital stay.
Pulmonary vascular resistance index and mortality after paediatric heart transplant
- Bryan G. Maxwell, Ahmad Y. Sheikh, Chinwe C. Ajuba-Iwuji, Eugenie S. Heitmiller, Luca A. Vricella
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- 23 September 2014, pp. 1141-1147
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Background: Although some prior studies have provided evidence to question the historical belief that pulmonary vascular resistance index ⩾6 Wood Units×m2 should be a contraindication to heart transplantation in children, no national analyses specific to the modern area have addressed this question. Methods: Data were analysed for paediatric heart transplant recipients from 1 January, 2002 to 1 September, 2012 (n=699). The relationship between pulmonary vascular resistance and all-cause 30-day mortality was evaluated using univariate and multivariate analyses. Results: The 30-day mortality included 10 patients (1.43%), which is lower than in the previous analyses. Receiver operating curve analysis of pulmonary vascular resistance index as a predictor of mortality yielded a cut-off value of 3.37 Wood Units×m2, but the area under the curve and specificity of this threshold was weaker than in previous analyses. Whereas pulmonary vascular resistance index treated as a dichotomised variable was a significant predictor of mortality in univariate (odds ratio 4.92, 95% confidence interval 1.04–23.33, p=0.045) and multivariate (odds ratio 5.26, 95% confidence interval 1.07–25.80, p=0.041) analyses, pulmonary vascular resistance index treated as a continuous variable was not a significant predictor of mortality in univariate (p=0.12) or multivariate (p=0.11) analyses. Conclusions: The relationship between pulmonary vascular resistance and post-heart transplant mortality in children is less convincing in this analysis of a comprehensive, contemporary database than in previous series. This suggests the possibility that modern improvements in the management of post-transplant right ventricular dysfunction have mitigated the contribution of pulmonary hypertension to early mortality.
Long-term outcome and anaesthetic management for non-cardiac surgery after Fontan palliation: a single-centre retrospective analysis
- Thomas Palumbo, Thierry Sluysmans, Jean E. Rubay, Alain J. Poncelet, Mona Momeni
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- 23 September 2014, pp. 1148-1154
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Objectives: The improved management of Fontan patients has resulted in good outcome. As such, these patients may necessitate care for non-cardiac surgery. We sought to determine the long-term outcome of our Fontan series palliated with the most recent surgical techniques. Our second objective was to report the incidence and the perioperative course after non-cardiac procedures. We reviewed the records of all patients with either a lateral tunnel or an extracardiac conduit Fontan between 1996 and 2008. Follow-up was recorded until June, 2013, including records regarding non-cardiac interventions. Results: Overall, 58 patients were included. Of them, one patient underwent a takedown of his Fontan, and five patients died in the immediate postoperative course. The cumulative survival of the remaining 52 patients was 81%. There was no significant difference in survival between right and left ventricle morphologies (p=0.56), nor between both types of Fontan (p=0.9). Chronic arrhythmias (25%), fatigue/dyspnoea (40%), thrombotic complications (19%), and embolic events (10%) were among the most recurrent comorbidities. In total, 45 non-cardiac interventions were performed on 26 patients, with three bleeding complications and one death. Conclusions: This study shows excellent long-term survival after both lateral tunnel and extracardiac conduit Fontan. The incidence of cardiovascular morbidity remains high, however. We also report a high number of non-cardiac interventions. Thorough understanding of the Fontan physiology is mandatory when non-cardiac anaesthesiologists are in charge of these patients.
Array comparative genomic hybridisation testing in CHD
- Hannah B. Hightower, Nathaniel H. Robin, Fady M. Mikhail, Namasivayam Ambalavanan
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- Published online by Cambridge University Press:
- 08 October 2014, pp. 1155-1172
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Background: CHD is the leading cause of mortality due to birth defects. Array comparative genomic hybridisation (aCGH) detects submicroscopic copy number changes and may improve identification of the genetic basis of CHD. Methods: This is a retrospective analysis of 1252 patients from a regional referral centre who had undergone aCGH. Of the patients, 173 had CHD. A whole-genome custom-designed oligonucleotide array with >44,000 probes was used to detect copy number changes. Results: Of the 1252 patients, 335 (26.76%) had abnormal aCGH results. Of the 173 patients with CHD, 50 (28.9%) had abnormal aCGH results versus 284 (26.3%) of 1079 non-cardiac patients. There were six patients with CHD who had well-described syndromes such as Wolf–Hirschhorn, trisomy 13, DiGeorge, and Williams. Of the patients with CHD, those with left-sided heart disease had the highest proportion (14/31; 45.13%) of abnormal aCGH results, followed by those with conotruncal heart disease (10/29; 34.48%), endocardial cushion defects (13/50; 26%), complex/other heart disease (12/52; 23.08%), and patent ductus arteriosus (1/11; 9.09%). Conclusions: Patients with CHD are at a substantial risk of having microdeletions and microduplications. The incidence of abnormalities on aCGH analysis is higher than identified with karyotype, and identification of copy number changes may help identify the genetic basis of the specific heart defects. However, aCGH may not have a significant diagnostic yield in those with isolated CHD. Further research using larger data sets may help identify candidate genes associated with CHD.
Echocardiographic assessment of left ventricular dyssynchrony in Egyptian children with congestive heart failure due to dilated cardiomyopathy
- Mohammed A. Al-Biltagi, Osama Abd Rab Elrasoul Tolba, Heba El Mahdy, Amr Donia, Shaimaa Elbanna
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- Published online by Cambridge University Press:
- 02 October 2014, pp. 1173-1181
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Objective: To evaluate the presence of cardiac dyssynchrony in Egyptian children with congestive heart failure due to dilated cardiomyopathy. Materials and methods: A total of 30 children with congestive cardiac failure due to dilated cardiomyopathy and 30 healthy age-matched controls were examined with conventional echocardiography, tissue Doppler, and speckle tracking imaging. Results: Conventional Doppler echocardiography demonstrated significant left ventricular systolic and diastolic dysfunction in the patient group. Tissue Doppler showed significant decrease in S-wave velocity and E'/A' ratio, and prolonged isovolumic contraction and relaxation times of mitral annulus as well as significant prolongation in mean difference between time-to-peak systolic strain of the basal septal and basal lateral segments in the patient group compared with the control group (p<0.005). Speckle tracking imaging demonstrated significant prolongation in mean difference between time-to-peak systolic strain of anteroseptal and posterior segments in both circumferential and radial strain analysis in the patient group than in the control group (p<0.005). It also demonstrated significant prolongation in the mean difference between time-to-peak systolic strain of the basal septal and basal lateral segments in longitudinal strain analysis in the patient group than in the control group (p<0.005). A significant increase in the standard deviation of time-to-peak strain, as a marker of increased intra-ventricular dyssycnrony, was present in the patient group compared with the control group (p=0.008). Conclusion: Children with congestive heart failure due to dilated cardiomyopathy usually suffer from significant intra-ventricular dyssynchrony. Tissue Doppler imaging and speckle tracking imaging strain analysis are helpful tools to detect the presence of cardiac dyssynchrony.